Results 1 to 10 of about 2,525 (118)

Paroxysmal nocturnal haemoglobinuria (PNH) manifesting on CT as a pathologic segment of small bowel [PDF]

Radiology Case Reports, 2017
Paroxysmal nocturnal haemoglobinuria, a rare, acquired, life-threatening disease of the blood, is characterised by a triad of haemolysis previously believed to occur mainly at night, bone marrow dysfunction, and thrombophilia.
David Zekria MPhys, DPhil, MBBS,, Peter Boavida BMedSci, BMBS, FRCR,   +1 more
exaly   +4 more sources

Paroxysmal Nocturnal Haemoglobinuria‐Associated Acute Cutaneous Thrombosis and Haemolysis in the Setting of Parvovirus B19 and Varicella Zoster Virus Infection [PDF]

eJHaem
A 28‐year‐old male with paroxysmal nocturnal haemoglobinuria (PNH) presented with headache, nasal bridge discomfort and haemoglobinuria. He developed a macular‐papular rash which rapidly progressed into purpura, necrosis and peri‐orbital oedema ...
Louise J. Potter, Roochi Trikha, Shreyans Gandhi, Joanne Large, Tanya N. Basu, Jeanne Boissiere, Hannah Devine, Taku Sugai, Aushna Rasool, Austin Kulasekararaj   +9 more
doaj   +2 more sources

Haploidentical allogeneic haematopoietic stem cell transplantation for paroxysmal nocturnal haemoglobinuria: a retrospective analysis [PDF]

Annals of Hematology
Objective: To evaluate the efficacy and prognostic factors of haploidentical haematopoietic stem cell transplantation (haplo-HSCT) for paroxysmal nocturnal haemoglobinuria (PNH).
Jing-Yi Bi, Yuan-Yuan Zhang, Hai-Xia Fu, Yun He, Ting-Ting Han, Yao Chen, Zhi-Dong Wang, Jun Kong, Jing-Zhi Wang, Xiao-Dong Mo, Yu-Qian Sun, Yi-Fei Cheng, Yu-Hong Chen, Yu Wang, Xiao-Hui Zhang, Xiao-Jun Huang, Zheng-Li Xu, Lan-Ping Xu   +17 more
doaj   +2 more sources

Haemolytic Anaemia; Beyond Sickle Cell Anaemia: A Case Series of Patients With Paroxysmal Nocturnal Haemoglobinuria in Kenya [PDF]

Case Reports in Hematology
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, acquired clonal haematopoietic stem cell disorder characterised by intravascular haemolysis, thrombosis and bone marrow failure.
Matilda Ong’ondi, Otom Lavender, Muraya Gathinji, Zaheer Bagha   +3 more
doaj   +2 more sources

Comparative Efficacy of Complement Inhibitors in Complement Inhibitor–Naïve PNH: A Systematic Review With Supportive Exploratory Network Meta‐Analysis of Randomized Trials [PDF]

eJHaem
Introduction Paroxysmal nocturnal haemoglobinuria (PNH) is an uncommon, life‐threatening disease, caused by intravascular haemolysis by the complement system.
Rehan Ishaque, Abdul Subhan Talpur, Hafiza Sidra, Huda Memon, Furqan Tarique Memon, Fawad Talat, Taha Rafiq, Supratik Rayamajhi   +7 more
doaj   +2 more sources

Anemia and Patient-Reported Outcomes in Patients With Paroxysmal Nocturnal Hemoglobinuria: A Real-World Observational Study. [PDF]

EJHaem
ABSTRACT Introduction Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder characterized by complement‐mediated hemolysis, thrombosis, bone marrow failure, and anemia. Although current treatments improve outcomes, patients still experience hemolysis and could remain anemic with a negative impact on quality of life (QoL).
Oliva EN, Risitano AM, Bona ED, Vannucchi AM, Dellfino IM, De Vivo A, Giai V, Notaro R, Iannì G, Beggiato E, Sica S, La Barba G, Balakrishnan S, Large J, Thavarajah T, Raso S, Fontana R, Barcellini W, Fattizzo B, Trikha R, Marano L, Panse J, Kulasekararaj AG.   +22 more
europepmc   +2 more sources

A machine learning algorithm for the detection of paroxysmal nocturnal haemoglobinuria (PNH) in UK primary care electronic health records [PDF]

Orphanet Journal of Rare Diseases
Background Paroxysmal Nocturnal Haemoglobinuria (PNH) is an ultra-rare, acquired disorder that is challenging to diagnose due to varied symptoms, heterogeneous patient presentations, and lack of awareness among healthcare professionals.
Amanda Worker, Hadley Mahon, Jack Sams, Freya Boardman-Pretty, Elena Marchini, Rand Dubis, Alan Warren, Jez Stockdale, Jyothika Kumar, Elizabeth Varones, Daniel Ollerenshaw, Calum Grant, Peter Fish, Richard J. Kelly   +13 more
doaj   +2 more sources

Thrombotic Events in Patients With Paroxysmal Nocturnal Haemoglobinuria Using Eculizumab: A Post‐Marketing Surveillance Sub‐Analysis [PDF]

eJHaem
Background Thrombotic events (TEs) account for approximately 40%–67% of deaths in untreated paroxysmal nocturnal haemoglobinuria (PNH). C5 inhibitor treatment (e.g., eculizumab and ravulizumab) greatly reduces the incidence of TEs in patients with PNH ...
Takayuki Ikezoe, Tatsuya Kawaguchi, Hideo Hayashi, Akihiko Shimono, Jun‐ichi Nishimura   +4 more
doaj   +2 more sources

Disease Activity After Switching From Eculizumab to Ravulizumab in Patients With Paroxysmal Nocturnal Haemoglobinuria: Real World Data From Denmark and Finland [PDF]

eJHaem
Introduction Ravulizumab replaced eculizumab as the preferred complement inhibitor for Paroxysmal Nocturnal Haemoglobinuria (PNH) in Denmark and Finland after 2020. Data on real‐world disease activity after switching are limited.
Anders Rønneholt‐Frederiksen, Dennis Lund Hansen, Vuokko Nummi, Jesper Stentoft, Ulrik M. Overgaard, Rie Sander Bech, Anna‐Elina Lehtinen, Andreas Glenthøj, Henrik Frederiksen   +8 more
doaj   +2 more sources

Role of NK cells in immune escape in patients with classical paroxysmal nocturnal haemoglobinuria [PDF]

Clinical and Translational Medicine
Background Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal haematopoietic stem cell disorder. Immune escape is crucial in PNH, and our previous studies revealed that natural killer (NK) cells potential participate in the immune escape of
Chaomeng Wang, Yan Yang, Wei Wang, Liyan Li, Mengting Che, Yingying Chen, Honglei Wang, Zhaoyun Liu, Lijuan Li, Hui Liu, Rong Fu   +10 more
doaj   +2 more sources